Diagnosis and Management of Pediatric Peripheral Neuropathies In Resource-Poor Settings

Abstract

The diagnosis of a peripheral neuropathy in a child who resides in the majority of resource-poor settings is based on the history taken and the clinical examination. The majority of children, unless they demonstrate additional clinical markers, will lack a more definitive diagnosis beyond the label ‘peripheral neuropathy’. The treatable, typically acquired conditions, which are prevalent in these settings, are the priority to identify. This would include neuroinfections, neuroinflammation, toxins and vitamin deficiencies. The management of children with peripheral neuropathies in resource-poor settings must be approached in a different manner to that of more ‘resource-equipped’ settings. Secondary or tertiary centers are scarce, often significant distances away from the patient, and this leads to long delays before access is possible. Most children present to primary healthcare settings and are seen by practitioners with little training in the features suggestive of a peripheral neuropathy. As such, basic aids to assist the healthcare worker in the early recognition and interventions of a child with a peripheral neuropathy are important. In addition, there must be recognition of the child with a rapidly progressive neuropathy where a life-threatening condition is present, and urgent referral to a tertiary setting made wherever possible.

Etiologic Spectrum of Biopsy-Proven Peripheral Neuropathies in Childhood from a Resource-Poor Setting

“…17 In the present day, the second largest group of heredodegenerative disorders with peripheral neuropathy can be diagnosed by specific metabolic or genetic testing without the need for a nerve biopsy. But in the absence of availability of these advanced tests, as in most developing countries, 6 nerve biopsy aids in definitive diagnosis, genetic counseling, and prognostication especially in patients with metachromatic leukodystrophy that forms a large proportion of cases in this category.…”

“…[3][4][5] Etiologic spectrum of peripheral neuropathies in the pediatric age group differ depending on the settings and the inclusion criteria. 6 In resource-poor settings, acquired causes dominate in contrast to higher prevalence of hereditary neuropathies in high-income countries. [7][8][9] Etiologic categories also differ when peripheral nerve biopsy is used as the entry point for analysis and varies with specific age groups.…”

“…2,11 The breakthroughs in genetic characterization of hereditary neuropathies in recent times is of limited use in developing countries because of limited access to these expensive tests. 6 Further, in resource-poor settings, nerve biopsies are often performed for diagnosis with a view to exclude acquired treatable disorders, that are more common in this setting. Nerve biopsy can be very informative in determining the basis of early-onset infantile neuropathies and specific neuropathologic abnormalities may point to particular genetic syndromes.…”

Prevalensi, Spektrum Klinis dan Gambaran Neurofisiologi Kasus Neuromuskular

“…Insiden SGB usia 0-15 tahun di Amerika latin, Amerika Serikat, Finlandia, dan Taiwan sebesar 0,34-1,34/100,000 anak. 7 Pada SGB, 90%-95% mengalami pemulihan dalam kurun waktu 6-12 bulan. 7 Sebagian kecil SGB akan berlanjut menjadi CIDP yang dapat mengalami perbaikan klinis yang lambat.…”

“…7 Pada SGB, 90%-95% mengalami pemulihan dalam kurun waktu 6-12 bulan. 7 Sebagian kecil SGB akan berlanjut menjadi CIDP yang dapat mengalami perbaikan klinis yang lambat. Perjalanan klinis DMD sangat lambat dan menyebabkan kematian di usia sekitar 20 tahun.…”